Rheumatic Heart Disease


Article Author:
Clarissa Dass


Article Editor:
Arun Kanmanthareddy


Editors In Chief:
Allison Castro


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
2/25/2019 6:49:13 PM

Introduction

Rheumatic heart disease is a systemic immune process that is sequelae to a beta-hemolytic streptococcal infection of the pharynx. It is most common in developing countries. However, it is responsible for 250,000 deaths in young people worldwide each year. Over 15 million people have evidence of rheumatic heart disease.[1]

Etiology

Rheumatic heart disease results from either single or repeated attacks of rheumatic fever that results in rigidity and deformity of valve cusps, the fusion of the commissures, or shortening and fusion of the chordae tendineae. Over 2 to 3 decades, valvular stenosis and/or regurgitation results. In chronic rheumatic heart disease, the mitral valve alone is the most commonly affected valve in an estimated 50 to 60% of cases. Combined lesions of both the aortic and mitral valves occur in 20% of cases. Involvement of the tricuspid valve occurs in about 10% of cases but only in association with mitral or aortic disease. Tricuspid valve cases are thought to be more common when recurrent infections have occurred. The pulmonary valve is rarely affected. 

Epidemiology

Rheumatic heart disease (RHD) is the most critical form of acquired heart disease in children and young adults living in developing countries. RHD accounts for approximately 15 to 20 percent of all patients with heart failure in endemic countries.[2]

A study of rheumatic heart disease cases estimated that in 2015, there were globally 33.4 million cases of RHD, 10.5 million disability-adjusted life-years due to RHD, and 319400 deaths due to RHD.[3] The incidence of rheumatic heart disease is highest in Oceania, central sub-Saharan Africa, and South Asia. In 2015, there was noted to be 3.4 cases per 100,000 population in nonendemic countries and 444 cases per 100,000 population in endemic countries.[3]

Rheumatic heart disease affects predominantly those living in poverty with inadequate access to health care and unchecked exposure to group A streptococcus. A systematic review and meta-analysis calculated the prevalence of clinically silent RHD (21.1 per 1000 people) to be approximately seven to eight times higher than that of clinically manifest disease (2.7 per 1000 people). Prevalence of rheumatic heart disease increases with age, from 4.7 per 1000 children at 5 years of age to 21.0 per 1000 children at 16 years of age.[4] Based on this data, estimates are that the RHD burden could increase by as much as double than that in the Global Burden of Disease study. Based on the fact that children in sub-Saharan Africa represent 6 to 7 percent of total global RHD burden, there may be an estimated 50 to 80 million persons currently affected with RHD worldwide.[5]

Pathophysiology

Rheumatic heart disease is the result of valvular damage caused by an abnormal immune response to Streptococcus pyogenes infection, which is classified as a group A streptococcus that causes acute rheumatic fever.[6]. Acute rheumatic fever occurs around three weeks after group A streptococcal pharyngitis that can affect joints, skin, brain, and heart.[7] After multiple episodes of rheumatic fever, progressive fibrosis of heart valves can occur, which can lead to rheumatic valvular heart disease. If valvular heart disease remains untreated, then heart failure or death may occur. The precise pathophysiology is not well known.[6]

History and Physical

Rheumatic fever is the primary cause of acquired heart disease in children and young adults worldwide. Rheumatic fever occurs 2 to 3 weeks after a group A beta-hemolytic streptococcal pharyngeal infection.[8]

Carditis is the most serious presentation of rheumatic fever. The symptoms and signs of carditis are dependent on the areas of the heart involved, which includes pericardium, myocardium or heart valves. The presentation of a pericardial friction rub on auscultation leans toward the diagnosis of pericarditis. The presence of signs of congestive heart failure points toward a diagnosis of myocarditis, which includes but not limited to lower extremity edema, shortness of breath with exertion or rest, abdominal distension, or inability to lay flat due to shortness of breath (orthopnea). Myocarditis in the absence of valvular disease is unlikely to be rheumatic in origin. Therefore, an apical systolic or basal diastolic murmur should be auscultated on physical exam. Mitral regurgitation is the most common valvular lesion, which is an apical pan-systolic murmur on auscultation.[8] Aortic regurgitation is less common. If patients have a known history of rheumatic heart disease, a change in the character of the murmur or presence of a new murmur on auscultation leads to the diagnosis of acute rheumatic heart fever. Rheumatic heart disease predominantly affects the left-sided cardiac valves.[8] The tricuspid valve and rarely pulmonary valve can be affected, but very unlikely without mitral valve involvement.

Evaluation

Rheumatic heart disease has a variety of clinical manifestations including myocarditis, decompensated congestive heart failure, arrhythmias (i.e., atrial fibrillation), and valvular heart disease. 

Myocarditis can result in conduction disturbances in the heart. Therefore, an EKG is necessary. An EKG can show varying forms of heart block including first degree, second degree or third degree AV block.

A chest x-ray should be completed to evaluate for cardiomegaly or pulmonary vascular congestion, which can be signs of congestive heart failure.

A transthoracic echocardiogram is more sensitive and specific than auscultation during physical examination for detection of rheumatic heart disease. Rheumatic heart disease seen on transthoracic echo without evidence of a murmur on auscultation is “subclinical rheumatic heart disease.”[8]

Mitral regurgitation is the most common presentation of rheumatic heart disease in young people. However, rheumatic heart disease is the most common cause of mitral stenosis worldwide.[9] Common descriptions of the mitral valve on echocardiography are ‘dog-leg’ ‘elbow’ or ‘hockey-stick’ deformities, which all help describe the thickening and restricted motion of the anterior mitral valve leaflet.[8]

In 2012, the World Heart Federation released criteria for echocardiographic diagnosis of rheumatic heart disease. The criteria are dependent on age and are broken down into older or younger than 20 years old.

Echocardiographic criteria for individuals aged less than or equal to 20 years old:

Definite Rheumatic Heart Disease (either A, B, C, or D):

A: Pathological mitral regurgitation and at least two morphological features of Rheumatic Heart Disease of the mitral valve

B: Mitral stenosis mean gradient greater than or equal to 4 mmHg

C: Pathological aortic regurgitation and at least two morphological features of rheumatic heart disease of the aortic valve

D: Borderline disease of both the aortic valve and mitral valve

Borderline Rheumatic Heart Disease (either A, B, or C):

A: At least two morphological features of rheumatic heart disease of the mitral valve without pathological mitral regurgitation or mitral stenosis

B: Pathological mitral regurgitation

C: Pathological aortic regurgitation

Normal echocardiographic findings (all four):

  1. Mitral regurgitation not meeting all four Doppler echocardiographic criteria (physiological mitral regurgitation)
  2. Aortic regurgitation not meeting all four Doppler echocardiographic criteria (physiological aortic regurgitation)
  3. An isolated morphological feature of rheumatic heart disease of the mitral valve (for example, valvular thickening) without any associated pathological stenosis or regurgitation
  4. The morphological features of rheumatic heart disease of the aortic valve (for example, valvular thickening) without any associated pathological stenosis or regurgitation

Echocardiographic criteria for individuals aged greater than 20 years old:

Definite Rheumatic Heart Disease (any one of the four):

  1. Pathological mitral regurgitation and at least two morphological features of rheumatic heart disease of the mitral valve
  2. Mitral stenosis mean gradient greater than or equal to 4 mmHg
  3. Pathological aortic regurgitation and at least two morphological features of rheumatic heart disease of the aortic valve, only in individuals aged less than 35 years
  4. Pathological aortic regurgitation and at least two morphological features of rheumatic heart disease of the mitral valve

Morphological Features of Rheumatic Heart Disease:

Mitral Valve:

  • Anterior mitral valve leaflet thickening greater than or equal to 3 mm (age-specific)
  • Chordal thickening
  • Restricted leaflet motion
  • Excessive leaflet tip motion during systole

Aortic Valve:

  • Irregular or focal thickening
  • Coaptation defect
  • Restricted leaflet motion
  • Prolapse

Pathologic mitral regurgitation (All four doppler criteria must be met):

  • Seen on two views
  • On at least one view jet length greater than or equal to 2cm
  • Peak velocity of greater than or equal to 3 meters/second
  • Pansystolic jet in at least one envelope

 Pathologic aortic regurgitation (All four Doppler criteria must be met):

  • Seen on two views
  • On at least one view jet length greater than or equal to 1cm
  • Peak velocity greater than or equal to 3 meters/second
  • Pandiastolic jet in at least one envelope

Treatment / Management

Management of rheumatic heart disease can be broken down into prevention and long-term management. Primary prevention of rheumatic heart disease centers on speedy recognition of treatment of group A streptococcal pharyngitis to prevent the development of acute rheumatic fever. Intramuscular benzathine penicillin G is the most widely used antibiotic to treat group A streptococcal pharyngitis.[10]

If a patient has a proven diagnosis of acute rheumatic fever, the goal of treatment is to suppress the inflammatory response to minimize the effects of inflammation on the heart and joints.  According to the World Heart Federation, the only cost-effective approach to preventing progression of rheumatic heart disease is secondary prophylaxis in the form of penicillin injections every 3 to 4 weeks to prevent recurrent group A streptococcal infection that causes recurrent episodes of acute rheumatic fever, which leads to progression of rheumatic heart disease.[10][8] The optimal duration of secondary prevention is unknown.[10]

Percutaneous mitral balloon valvuloplasty is the standard first-line therapy for cases of rheumatic mitral stenosis in the absence of regurgitation, arrhythmias and left atrial thrombus.[9] Surgical intervention is commonly the mainstay of treatment in severe cases of valvular disease. Surgical intervention can either be valve replacement or repair. 

If a patient develops heart failure due to valve disease, the patient should be placed on medical therapy as tolerated for heart failure including ACE inhibitors, diuretics, and beta blockers.

At this time, there is no cure of rheumatic heart disease.

The physician should also have appropriate surveillance on patients diagnosed with rheumatic fever and rheumatic heart disease to allow for initiation of appropriate therapy as early as possible.

Differential Diagnosis

  • Endocarditis
  • Viral myocarditis
  • Functional mitral regurgitation associated with viral illness, such as viral myocarditis
  • Mitral valve prolapse due to degenerative myxomatous disease 

Prognosis

Rheumatic heart disease causes at least 200000 to 250000 premature deaths every year.[6] It is also the major cause of cardiovascular death in children and young adults in countries that have poor medical attention. If the valvular disease is not serially monitored, the patient may not present until severe heart failure is present resulting in surgical contraindication.

Complications

Rheumatic heart disease is generally latent or silent until cardiac complications develop in late adulthood. Infective endocarditis, an embolic event, heart failure, pulmonary hypertension, and atrial fibrillation from untreated severe valvular disease are the most common complications.[10]

Deterrence and Patient Education

Rheumatic heart disease is highly prevalent in developing countries leading to the cause of the most cardiovascular morbidity and mortality in young people. Guidelines provided by the World Health Federation have outlined that the most cost-effective way to prevent rheumatic heart disease is with antibiotic prophylaxis after the diagnosis of rheumatic fever. Patients should be informed of their diagnosis of rheumatic fever as well as the complications associated with poor compliance with antibiotics and follow-up including valvular heart disease, heart failure, and arrhythmias.

Enhancing Healthcare Team Outcomes

Primary care physicians and nurse practitioners who have patients with a known diagnosis of rheumatic fever should maintain and stress the importance of strict follow-up with the patients.  To prevent rheumatic heart disease, patients should undergo close monitoring with complete history and physicals and transthoracic echocardiography. Patients should be referred to cardiology if they start developing a new murmur, signs, and symptoms of heart failure, arrhythmia, or evidence of valvular disease on transthoracic echocardiography.  Surgery is often required when the mitral and/or aortic valve is severely damaged.


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Rheumatic Heart Disease - Questions

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The pathologist reports that Aschoff nodules were found. What was the most likely organ examined?



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Which heart valve is most often affected by acute rheumatic fever?



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In acute rheumatic fever, which heart valve is involved second most commonly?



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What disease is characterized by MacCallum plaques?



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After infection of the throat, how long does it take rheumatic fever to cause symptoms of mitral stenosis?



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The pathologist reports the presence of McCallum plaques. This means that the patient may have which disease?



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Which of the following is not a major criterion for rheumatic fever?



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Typical Aschoff nodules are composed of which of the following?



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A patient presents with anterior chest pain and has a cardiac friction rub without murmurs. Two weeks ago the patient had a sore throat with a fever to 100 degrees F and a skin rash. What test should be performed?



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A 7-year-old girl develops migratory arthritis that first involved the left knee and then the right elbow. There is a history of pharyngitis two weeks ago. On physical examination, the child is febrile with a temperature of 102 degrees F (38.8 C). The right elbow is red, swollen, and tender with movement. Lung and cardiovascular examinations are normal. The erythrocyte sedimentation rate is 60 ml/hr. Which of the following is the most likely diagnosis?



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A diastolic rumble is auscultated at the apex on cardiac exam of a 73-year-old female who presents with dyspnea on exertion and heart palpitations. What is the most likely diagnosis?



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Which of the following would not be indicative a new diagnosis of rheumatic fever?



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A 20-year-old patient from the Ukraine presents for follow-up after hospitalization for a new onset of atrial fibrillation. As a child, she had an illness that caused 1 month of uncontrollable choreiform movements of her arms and legs. Subsequently, she had two episodes of large joint arthritis, which resolved with medications. Current medications include warfarin and metoprolol. A cardiac exam shows an irregularly irregular rate with a soft diastolic and 3/6 holosystolic murmur at the apex. The patient is referred for an ECG to determine the necessity of a valve replacement. Which additional intervention would be the most appropriate?



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A 10 year old girl presents with chorea of the hand. On examination, she has milkmaid sign and darting tongue. Which of the following investigations should be done?



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Which of the following findings is associated with rheumatic fever?



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A child developed swelling and tenderness of his left knee. The next day his left knee was fine, but he developed similar symptoms on his right wrist. Which of the following is the most likely diagnosis?



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What would be the best initial treatment of acute rheumatic fever?



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A patient is diagnosed with a calcific valvular abnormality at age 60. As a child, he had a severe infectious illness with arthritis. Which of the following was the most likely cause?



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What is the incidence of acute rheumatic fever (ARF) from untreated streptococcal pharyngitis?



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Which heart valve is most commonly affected by acute rheumatic fever?



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A heart murmur from acute rheumatic fever is most likely the result of which of the following?



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Which of the following is the most common finding in patients with rheumatic fever?



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What is the cause of rheumatic fever?



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Which of the following is a required Jones criteria for the diagnosis of acute rheumatic fever?



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The most common combination of multiple valve involvement in chronic rheumatic valvulitis is:



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A patient with a history of rheumatic fever is seen in the clinic because of ongoing shortness of breath for the past 3 months. He appears emaciated and auscultation reveals a loud S1 and a diastolic rumble. What classic feature of the heart disorder will the patient have on a chest x-ray?



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A 9-year-old child has been diagnosed with acute rheumatic fever. Which heart structures are at risk of being damaged? Select all that apply.



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A patient has been admitted with a diagnosis of acute rheumatic fever. What is true regarding acute rheumatic fever? Select all that apply.



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Rheumatic Heart Disease - References

References

Watkins DA,Beaton AZ,Carapetis JR,Karthikeyan G,Mayosi BM,Wyber R,Yacoub MH,Zühlke LJ, Rheumatic Heart Disease Worldwide: JACC Scientific Expert Panel. Journal of the American College of Cardiology. 2018 Sep 18;     [PubMed]
Marijon E,Mirabel M,Celermajer DS,Jouven X, Rheumatic heart disease. Lancet (London, England). 2012 Mar 10;     [PubMed]
Liu M,Lu L,Sun R,Zheng Y,Zhang P, Rheumatic Heart Disease: Causes, Symptoms, and Treatments. Cell biochemistry and biophysics. 2015 Jul;     [PubMed]
Reményi B,Wilson N,Steer A,Ferreira B,Kado J,Kumar K,Lawrenson J,Maguire G,Marijon E,Mirabel M,Mocumbi AO,Mota C,Paar J,Saxena A,Scheel J,Stirling J,Viali S,Balekundri VI,Wheaton G,Zühlke L,Carapetis J, World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline. Nature reviews. Cardiology. 2012 Feb 28;     [PubMed]
Nulu S,Bukhman G,Kwan GF, Rheumatic Heart Disease: The Unfinished Global Agenda. Cardiology clinics. 2017 Feb;     [PubMed]
Watkins DA,Johnson CO,Colquhoun SM,Karthikeyan G,Beaton A,Bukhman G,Forouzanfar MH,Longenecker CT,Mayosi BM,Mensah GA,Nascimento BR,Ribeiro ALP,Sable CA,Steer AC,Naghavi M,Mokdad AH,Murray CJL,Vos T,Carapetis JR,Roth GA, Global, Regional, and National Burden of Rheumatic Heart Disease, 1990-2015. The New England journal of medicine. 2017 Aug 24;     [PubMed]
Bocchi EA,Guimarães G,Tarasoutshi F,Spina G,Mangini S,Bacal F, Cardiomyopathy, adult valve disease and heart failure in South America. Heart (British Cardiac Society). 2009 Mar;     [PubMed]
Rothenbühler M,O'Sullivan CJ,Stortecky S,Stefanini GG,Spitzer E,Estill J,Shrestha NR,Keiser O,Jüni P,Pilgrim T, Active surveillance for rheumatic heart disease in endemic regions: a systematic review and meta-analysis of prevalence among children and adolescents. The Lancet. Global health. 2014 Dec;     [PubMed]
Weinberg J,Beaton A,Aliku T,Lwabi P,Sable C, Prevalence of rheumatic heart disease in African school-aged population: Extrapolation from echocardiography screening using the 2012 World Heart Federation Guidelines. International journal of cardiology. 2016 Jan 1;     [PubMed]
Seckeler MD,Hoke TR, The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease. Clinical epidemiology. 2011 Feb 22;     [PubMed]

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The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Nurse-Microbiology. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Nurse-Microbiology, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Nurse-Microbiology, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Nurse-Microbiology. When it is time for the Nurse-Microbiology board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Nurse-Microbiology.